Materiały źródłowe

• #1 Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9244941/

  Myxofibrosarcoma (MFS) is a common entity of adult soft tissue sarcomas (STS) characterized by a predilection of the extremities and a high local recurrence rate. […] Clinical manifestation of MFS is characterized by slowly enlarging and painless mass with high propensity for local recurrence. […] Myxofibrosarcoma is characterized by the highest rate of recurrence among all STS, ranging from 20-60% after 5 years, depending on the case series. […] Low-grade diseases exhibit a high rate of multiple local recurrences and low rate of metastatic potential. Instead, high-grade lesions show higher metastatic potential. […] The overall incidence of distant metastases is estimated to be between 20% and 25%. […] Currently, histological MFS grading relies on the general FNCLCC grading system for STS, which assigns grades 1 to 3 depending on tumor differentiation, mitotic count and tumor necrosis.

• #2 Myxofibrosarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/myxofibrosarcoma/

  Myxofibrosarcoma is a malignant fibroblastic neoplasm characterized by diverse cellularity, myxoid stroma, and curvilinear vasculature, commonly affecting elderly patients. […] Most patients present with a slowly enlarging and painless mass. […] Myxofibrosarcoma is one of the most common sarcomas of elderly patients, with a slight male predominance. Although the overall age range is wide, these neoplasms mainly affect patients in the sixth to eighth decades of life, whereas they only exceptionally rarely arise in patients aged < 30 years. [...] Local, often repeated recurrences, unrelated to histological grade, occur in 30–40% of cases, usually as a result of inadequate surgery, sometimes even in experienced hands. Metastases and tumor-related mortality are closely related to tumor grade. The overall 5-year mortality rate is 30–35%. Although none of the low-grade neoplasms metastasize, high-grade tumors develop metastases in 20–35% of cases. In addition to pulmonary and osseous metastases, lymph node metastases are sometimes seen. Local recurrence within 12 months increases tumor-associated mortality. Given the propensity for relentless local recurrence associated with highly infiltrative growth, aggressive surgery combined with radiotherapy is advised for myxofibrosarcoma in order to achieve improved local control, which may translate into survival benefits. Compared with other high-grade pleomorphic sarcomas of somatic soft tissue, intermediate-grade and high-grade myxofibrosarcomas have a lower metastatic rate. tumor size, morphological grade (inversely related to the percentage of the myxoid component), and surgical margins are significant predictors of survival. The epithelioid subtype of myxofibrosarcoma behaves more aggressively, with an increased risk of metastasis (> 50%).

• #3 Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation | Anticancer Research

  https://ar.iiarjournals.org/content/39/6/2985

  Myxofibrosarcoma (MFS) represents approximately 20% of all soft-tissue sarcomas, especially in elderly patients in their sixth to eighth decades of life. […] MFS constituted the most common high-grade sarcoma (G2/G3, 79.4%) in our cohort and was generally located in a lower limb (73.6%). […] Local recurrence occurred in 23.5% of all patients after a mean disease-free period of 19.4 months. […] Metastasis occurred after a mean of 20.4 months in 38.2% of the patients. […] Tumour grade and metastasis were significant prognostic factors of survival (log-rank test p=0.041 and p=0.00007). […] Ten patients (29.4%) died due to MFS during our follow-up period. […] The survival rate was significantly affected by the tumour grade and presence of metastases. Patients with metastases had significantly worse overall survival than patients without metastases (hazard ratio=2.97, p=0.005) and than patients with G3 MFS (hazard ratio=1.56, p=0.024). […] The occurrence of metastasis significantly negatively correlated (log-rank test p=0.00007) with patient survival. […] The survival rate was highly dependent on the tumour grade, the achievement of negative margins during surgery, and metastasis.

• #4 Myxofibrosarcoma: Prognosis, Treatment & Staging

  https://my.clevelandclinic.org/health/diseases/22563-myxofibrosarcoma

  Myxofibrosarcoma (MFS) is a type of soft tissue sarcoma (cancer). It starts as a small lump. As the lump grows, you may have pain or swelling. MFS is an aggressive tumor, meaning it spreads (metastasizes) quickly to other parts of your body. Its more likely to grow back (recur) after treatment than some other types of cancers. […] You may not have any symptoms of myxofibrosarcoma when it first appears. As the tumor grows, you may notice a lump beneath your skin. The lump is often painless, but you may have discomfort or swelling. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. Low-grade myxofibrosarcoma is less likely to return than higher-grade tumors.

• #5 Myxofibrosarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/myxofibrosarcoma/symptoms-causes/syc-20577507

  Myxofibrosarcoma starts as a growth of cells that can grow into healthy body tissue. Myxofibrosarcoma might cause a lump under the skin that grows slowly. It often isn’t painful. […] Symptoms of myxofibrosarcoma include: A painless lump on an arm or leg. A lump that grows slowly. […] Myxofibrosarcoma most often starts in the leg. It rarely starts in the abdomen, where other kinds of sarcomas tend to grow.

• #6 Myxofibrosarcoma | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/myxofibrosarcoma

  Myxofibrosarcoma might cause a lump under the skin that grows slowly. It often isn’t painful. […] Symptoms of myxofibrosarcoma include: A painless lump on an arm or leg. A lump that grows slowly. […] Myxofibrosarcoma most often starts in the leg. It rarely starts in the abdomen, where other kinds of sarcomas tend to grow.

• #7 Myxofibrosarcoma: Causes, Symptoms, and Treatment Options

  https://sehathub.com/myxofibrosarcoma

  The symptoms of MFS depend on the location and origin of the tumor. Most MFS tumors develop in the subcutaneous tissues, while others infiltrate the underlying fascia or skeletal muscle. Early on, symptoms may be absent or minimal. Over time, a painless, slow-growing lump may appear under the skin. In some cases, patients experience discomfort, localized swelling, or systemic inflammatory responses. […] MFS represents a slow-growing or painless mass that usually occurs under the skin of the legs and arms. It often exhibits an infiltrative growth pattern, which increases the risk of local recurrence even after surgical removal. In some cases, multiple tumor nodules may form within the affected region, and while MFS primarily spreads locally, distant metastases most commonly to the lungs can occur.

• #8 Myxofibrosarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/myxofibrosarcoma/

  Myxofibrosarcoma is a type of soft tissue sarcoma, which is a type of cancer. It affects the bodys fibrous connective tissues. These tissues support bones, muscles, and organs and holds them in place. Symptoms can vary depending on the size and location of your tumour. The most common symptom is a lump or swelling, which is often under the skin of the arms or legs, is usually painless, and often grows slowly. Myxofibrosarcoma can come back after treatment. If it reappears in the same area as the original tumour, its known as a local recurrence. If your cancer returns, it can be accompanied by cancer in other parts of your body. This is called metastasis or secondary cancer. In people with myxofibrosarcoma, these secondary cancers can appear in the lungs, which is why a chest x-ray is taken at follow-up appointments. […] If the cancer does reappear, its important to get treated quickly. You may have surgery and/or radiotherapy. Your treatment plan will be determined by your doctor.

• #9

  https://www.sydneysarcomaunit.com.au/myxofibrosarcoma

  The symptoms for Myxofibrosarcoma include: […] Formation of a lump or mass, which might be painful in nature. […] Symptoms depend on where in your body the mass develops and its effect on local structures.

• #10 Myxofibrosarcoma – Symptoms, Treatment & Support – Without a Ribbon

  https://withoutaribbon.org/myxofibrosarcoma-symptoms-treatment-support/

  Myxofibrosarcoma is a rare, slowly growing aggressive tumor that occurs in the soft tissues of the body. […] Signs and symptoms depend upon the location of the tumor. […] Most of the small myxofibrosarcoma tumors are painless. […] Large myxofibrosarcoma tumors can cause pain or disturbance in the function of the organ. […] In advanced cases symptoms of fever, weight loss and night sweats can be observed.

• #11 Myxofibrosarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/myxofibrosarcoma-causes-symptoms-and-treatment?srsltid=AfmBOooa2Kp23yDzetsp0Ply3Cq8E3y9fhsIbsYaEIsbYGk1F9gco91a

  Myxofibrosarcoma usually does not showcase any characteristic symptoms in the initial stage. […] Symptoms in the advanced stage include: […] An obvious lump or swelling under the skin tissue […] Intense pain, if the tumour presses on any nerves or muscles. […] Myxofibrosarcoma is quite a rare form of cancer, making up only 2 per cent of all cancer diagnoses. […] Being an aggressive form of cancer, MFS rapidly metastasizes to other parts of the body, affecting vital organs. […] Unlike other forms of cancer, Myxofibrosarcoma tends to recur or grow back even after complete treatment.

• #12 Giant Myxofibrosarcoma in the Lower Limb: An Overview of Diagnostic and Clinical Management

  https://www.mdpi.com/2075-4418/14/12/1298

  Myxofibrosarcoma (MFS), an aggressive soft tissue sarcoma, is one of the undifferentiated pleomorphic sarcomas; it has a low incidence, affecting people in the sixth to eighth decades of life. It usually involves the extremities and is painless with a slow-growing pattern. […] MFS commonly affects people in the sixth to eighth decades of life, having a slight predisposition in male patients. […] These tumors are normally painless with a slow-growing pattern and are classified as superficial (affecting the dermal and subcutaneous layers) and deep (involving muscular and subfascial tissues). […] Patients with MFS usually present a painless mass that has a slow size increase, with 30–60% of tumors being deeply located. Symptoms are normally related to the anatomical area of origin. […] MFS usually affects the extremities, as in our case, and rarely the trunk, the head, and the neck, and is initially asymptomatic.

• #13 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma?trendmd-shared=0

  Presence of a slowly-growing mass is observed; no pain is generally noted. […] The tumors may be sited just below the skin surface or in the deep tissues. 50-65% of the tumors affect the skin/subcutaneous region; the remaining 35-50% are found deeper in the tissues or muscles. […] Tumors just below skin are often multinodular in appearance; those that are deeper are often present as a single infiltrating mass. Sometimes, the extent of tumor spread may be difficult to determine due to severe tumor infiltration. […] Most tumors affect the arms and legs (especially the limb girdle); legs are affected more than the arms. However, hands and feet are only rarely affected. […] Rarely, Myxofibrosarcoma have been observed on the chest and back (trunk), head and neck region. Abdominal or retroperitoneal tumors are also seen very rarely. […] Note: Myxofibrosarcomas may be low-grade, intermediate-grade, or high-grade. The higher the tumor grade, the greater is the chance of infiltrative behavior and metastasis (spread to other body parts). Low-grade Myxofibrosarcoma tumors are not known to metastasize.

• #14 Soft tissue sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725

  A soft tissue sarcoma may not cause any symptoms at first. As the cancer grows, it may cause: […] A noticeable lump or swelling. […] Pain, if the growth presses on nerves or muscles.

• #15

  https://winshipcancer.emory.edu/cancer-types-and-treatments/sarcoma/diagnosis.php

  Both soft tissue and bone sarcomas can present in multiple ways from a slowly growing painless mass to one that is rapidly enlarging. Therefore, signs and symptoms can range from mild to severe, depending on location, the size of the tumor and the stage. Examples include: […] A new lump or bump present underneath the skin […] A lump or bump that appears to grow in size (any mass that is larger than 3 centimeters should be evaluated by a doctor) […] Increasing abdominal girth […] Pain in and around a bone for no apparent reason […] Swelling around a bone or joint […] Inability to move or lift a limb.

• #16 Sarcoma: What it Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17934-sarcoma

  Symptoms vary depending on the tumors location. For example, some sarcomas may not cause noticeable symptoms in the early stages. Some sarcomas may feel like a painless lump under your skin, while others dont cause pain until they grow large enough to press on an organ. […] Other sarcomas can cause long-lasting bone pain or swelling in your arm or leg that worsens at night. These changes may limit your movement. […] Symptoms may include: A new lump that may or may not hurt. Pain in an extremity (arm/leg) or abdomen/pelvis. Trouble moving your arm or leg (a limp or limited range of motion). Unexplained weight loss. Back pain. […] Sarcoma shares symptoms with many other conditions. Your provider can help determine whether your symptoms are related to sarcoma or another disease or disorder.

• #17 Soft Tissue Sarcoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/soft-tissue-sarcoma-causes-symptoms-and-treatment?srsltid=AfmBOopHABZ3I7Q45y7nPKIWUGqM7EzIF12mNTK8jXLuNu27sQCfysCS

  The characteristic signs and symptoms of Soft tissue sarcoma include: […] Formation of a new, noticeable, painless lump anywhere on the body […] Swelling under the skin that grows larger with time and hurts […] Formation of a lump near the lungs that might cause incessant cough or a feeling of breathlessness […] Pain in different parts of the body arising due to the pressure from tumours on the nerves […] Swelling in the stomach […] Abdominal pain […] Constipation […] Feeling full all the time […] Excessive fatigue […] General debility […] Nausea and vomiting […] Sudden weight loss without trying.

• #18 Internet Scientific Publications

  https://ispub.com/IJS/28/3/14130

  A 56-year-old male visited a local physician with intermittent abdominal pain. […] Clinically, a freely mobile mass was palpable in the right para-umbilical region. […] Ultrasound-guided fine-needle aspiration cytology (FNAC) showed features of a spindle cell tumor. […] Histopathological examination revealed myxoid areas with pleomorphic stellate and spindle cells. […] The nucleoli were pleomorphic and hyperchromatic with prominent nucleoli. […] Many branching vessels were seen throughout with bizarre multinucleate giant cells. […] Features were consistent with high-grade myxofibrosarcoma. […] Retroperitoneal tumors typically have vague presenting symptoms. […] These tumors are provided with a well-concealed, widely expansible area, leading to the development of large masses with local and distant metastases before the patient becomes symptomatic.

• #19 Low grade myxofibrosarcoma in the right ventricle presenting as pulmonary thromboembolism – Suh – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/17462/html

  We report an unusual malignant cardiac neoplasm that initially presented as pulmonary thromboembolism in a 78-year-old male. […] Despite anticoagulation, the pulmonary artery lesion progressed and a mass-like lesion developed in the right ventricular outflow tract. […] The patient was admitted 6 months later with aggravated dyspnea. […] Echocardiography showed dilated right atrium and right ventricle and severe right ventricular dysfunction with severe tricuspid regurgitation, and the systolic pulmonary artery pressure exceeded 80 mmHg. […] The low-density lesion was again noted in the right ventricle, and other low-density lesions in the pulmonary artery had progressed to extend into both pulmonary arteries and the right descending interlobar and lobar pulmonary arteries in chest computed tomography.

• #20 Primary cardiac myxofibrosarcoma of the left atrium and pericardium: a case report | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-023-02441-9

  Primary cardiac myxofibrosarcoma is rare and commonly occurs in the left atrium. Myxofibrosarcoma is aggressive and has a high mortality rate due to its high rate of recurrence. Complete surgical resection is considered important; however, effective treatment options have not been established. […] The clinical presentation often varies. Similar to other benign or malignant cardiac tumors, cardiac myxofibrosarcoma presents with symptoms such as dyspnea, chest pain, edema, and syncope. The LA is the most common location of the tumor, followed by the LA+PV, right heart system, and left ventricle. […] The median survival time of primary cardiac myxofibrosarcoma patients treated with tumor resection is only 14 months. Although complete surgical resection is important for prolonged survival, it is difficult, and only occurs in one-third of cases.

• #21

  https://crimsonpublishers.com/tnn/fulltext/TNN.000530.php

  Myxofibrosarcoma is a common sarcoma affecting extremities of elderly age group. It rarely affects the younger age group and the head neck region. It exhibits an infiltrative growth pattern and has a high local recurrence rate. […] A 14-year-old boy presented with a one-year history of a painless, progressive swelling over the left occipital region. He denied constitutional symptoms and was neurologically intact on examination. […] Pathological evaluation of the surgical specimen revealed a low grade Osteosarcoma. Immunohistochemistry clinched the diagnosis of a myxofibrosarcoma. […] Myxofibrosarcoma is the most common sarcoma in the elderly age group. Its peak incidence is in the seventh decade with slight male preponderance. It exhibits a slow and painless progression. […] The tendency of myxofibrosarcoma to be of progressively higher grade in recurrences by becoming gradually more cellular, more pleomorphic, more mitotically active, and more necrotic seems to be a distinctive feature and is in contrast to most other sarcomas.

• #22 Giant Myxofibrosarcoma in the Lower Limb: An Overview of Diagnostic and Clinical Management

  https://www.mdpi.com/2075-4418/14/12/1298

  There are reported cases with paraneoplastic secretion of insulin-like growth factor (IGF) causing hypoglycemic attacks in patients with myxofibrosarcoma. In these patients, hypoglycemia ceased after the surgical removal of the tumor or after chemotherapy and radiotherapy. […] Myxofibrosarcoma remains a difficult disease to diagnose and treat properly, with high morbidity; therefore, our recommendation for such a case, in accordance with the current standard of care, is to be referred to experienced centers where a multidisciplinary team can increase the likelihood of success.

• #23 Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model | Modern Pathology

  https://www.nature.com/articles/3800550

  Myxofibrosarcoma is one of the most frequent soft tissue tumours in elderly patients, mostly arising in the extremities. Grade I lesions are only locally aggressive whereas grade II and grade III lesions have metastatic potential. […] Local recurrences showed increase in grade compared to their primary lesions. Interestingly, these recurrences showed more complex cytogenetic aberrations. Increase in grade seems to parallel increase in cytogenetic aberrations and malignant potential. […] According to previous studies, grade II and III tumours are true malignant tumours since they have metastatic potential. This is in contrast to grade I lesions, which are only locally aggressive. However, since local recurrence goes together with increase in grade, grade I myxofibrosarcoma may finally gain metastatic potential, warranting close surveillance.

• #24 Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives

  https://www.mdpi.com/2075-4418/13/19/3022

  Myxofibrosarcoma (MFS) is clinically characterized by a high risk of local recurrence related to its infiltrative growth pattern. Local recurrences occur in 10–61% of cases, irrespective of histological grade. Notably, 19–50% of locally recurrent MFS cases progress to a higher histological grade with an attendant increase in metastatic potential. Local recurrence within 12 months of initial surgery is associated with a higher mortality rate. Low-grade MFS has no metastatic potential, whereas intermediate- and high-grade MFSs develop distant metastases in 16–38% of cases. A recent epidemiological study from the Netherlands comprising 908 MFS patients indicates that median overall survival (OS) is 155 (range 0.1–215) months, with a 5-year OS of 67.7%. The 5-year OS of MFS is better when compared to other types of STS. It is currently recognized that age, sex, tumor size, histological grade, and surgical margins are the significant prognostic factors for MFS. Moreover, the presence of systemic inflammation has recently been reported to be associated with a worse prognosis in patients with high-grade MFS. […] High-grade MFS displays highly complex karyotypes with multiple copy number alterations. The progression in grade is accompanied by an increase in cytogenetic alterations. In general, high-grade MFS shows a higher amount of somatic copy number alterations than low-grade MFS.

• #25 Analysis of prognostic factors in 171 patients with myxofibrosarcoma of the trunk and extremities: a cohort study

  https://atm.amegroups.org/article/view/77255/html

  Myxofibrosarcoma (MFS) of the trunk and extremities has unique clinical features. […] The 3-year recurrence, 3-year metastasis, and 5-year survival rates were 29.2%, 19.3%, and 93.6%, respectively. […] The surgical margin and tumor size were associated with recurrence. […] The surgical margin and CD44 expression were associated with metastasis. […] A negative surgical margin can be reduced effectively the rate of recurrence and metastasis in patients with MFS of the trunk and limbs. […] The 3-year recurrence rate was 29.2%, and 33 cases had metastasis. […] The multivariate analysis showed that the surgical margin and tumor size were risk factors for local recurrence. […] In addition, the surgical margin and presence of CD44 were risk factors for distant metastasis. […] The results showed that the 20 patients with positive incision margins had a 3-year recurrence rate of 65%, which was higher than the 24.5% recurrence rate in the negative incision margin group. […] The multivariate analysis showed that the surgical margin was an independent risk factor for recurrence and metastasis within 3 years. […] The larger the tumor, the higher the recurrence rate.

• #26 Prognostic Factors and Outcomes of Patients with Myxofibrosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3837421/

  Myxofibrosarcomas (MFS) are a historically heterogeneous group of tumors that exhibit a propensity for local recurrence. […] MFS exhibit a propensity for local recurrence, which is predicted by resection with positive or close margins. […] Myxofibrosarcoma (MFS) is a malignant mesenchymal tumor that usually arises in the extremities of elderly patients. […] This penchant for local recurrence is concerning, given that up to 50 % of recurrences exhibit higher-grade histology and correspondingly greater metastatic potential than the primary lesion, postulated to be related to the infiltrative growth pattern seen in MFS. […] Local recurrence occurred in 11 patients (16 %) at a median of 16 months (range 685 months) after diagnosis. […] Close/positive margin status (HR 4.34, P = 0.030) was the sole significant predictor of local recurrence on univariate analysis, while R status (HR 3.08, P = 0.064) showed a trend toward significance.

• #27 Internet Scientific Publications

  https://ispub.com/IJS/28/3/14130

  Todd et al. reported that the most common clinical picture of retroperitoneal sarcoma cases at presentation includes back pain and weight loss (37.5% of patients with either symptom), with fatigue (25%), increased abdominal girth (12.5%), and fever or night sweats (12.5%) also noted. […] These patients exhibited symptoms for an average 3.5 months before presentation. […] The diagnosis of retroperitoneal tumor is aided by imaging studies which contribute greatly to delineating the size, location, and character of a mass. […] A myxofibrosarcoma on CT and magnetic resonance imaging typically has uneven contrast-enhanced effects with both solid and cystic components. […] The tendency of myxofibrosarcoma to be of progressively higher grade in recurrences by becoming gradually more cellular, more pleomorphic, more mitotically active, and more necrotic seems to be a distinctive feature and is in contrast to most other sarcomas.

• #28 Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model | Modern Pathology

  https://www.nature.com/articles/3800550

  We showed that local recurrences occurred in all grades, with increase in grade upon recurrence, which is in accordance to the literature. […] Recurrences showed increase in grade and more complex aberrations than the primary tumours. These complex aberrations suggest additional chromosomal events, corresponding with an increase in histological grade and, subsequently, increase in malignant potential.

• #29 Myxofibrosarcoma | Oncohema Key

  https://oncohemakey.com/myxofibrosarcoma/

  Myxofibrosarcoma is a unique subtype of soft tissue sarcoma with a locally infiltrative behavior and a predilection for local recurrence. […] A subset of patients with higher-grade lesions will develop distant metastases. […] Myxofibrosarcoma can progress to a completely cellular neoplasm devoid of a myxoid component that is indistinguishable from UPS. […] There is a tendency for myxofibrosarcomas to become more cellular over time and with local recurrence, suggesting this is a form of tumor progression in at least some cases. […] Neoplasms composed mainly of the hypocellular myxoid component tend to show local recurrence rather than distant metastasis.

• #30 Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model

  https://lirias.kuleuven.be/775149

  Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model […] Myxofibrosarcoma is one of the most frequent soft tissue tumours in elderly patients, mostly arising in the extremities. Grade I lesions are only locally aggressive whereas grade II and grade III lesions have metastatic potential. […] Local recurrences showed increase in grade compared to their primary lesions. Interestingly, these recurrences showed more complex cytogenetic aberrations. Increase in grade seems to parallel increase in cytogenetic aberrations and malignant potential. […] Based on these findings, we suggest that tumorigenesis of myxofibrosarcoma is mainly a multistep genetic process, probably ruled by genetic instability caused by targeted checkpoint genes.

• #31 Myxofibrosarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Myxofibrosarcoma

  MFS metastasize most commonly to the lungs, bone, and lymph-nodes. […] Individuals with the epithelioid variant of FBS generally present with a tumor in the limbs; the tumors tend to be somewhat larger, more aggressive, and more likely to metastasize than the tumors in non-variant cases. At least 50% of patients with this variant have developed metastases. […] The recommended treatment for individuals presenting with localized MFS tumors is radical surgical resection. […] Historically, about 10% of patients treated with radical surgery developed recurrences at the surgical site and a significant number of these patients developed metastatic disease.

• #32 Primary cardiac myxofibrosarcoma of the left atrium and pericardium: a case report | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-023-02441-9

  Myxofibrosarcoma is categorized as a low grade to high grade neoplasm. Low grade myxofibrosarcoma tends to metastasize locally, whereas high grade myxofibrosarcomas invade local and adjacent tissues and are associated with distant metastasis to the lung, bone, brain, and lymph nodes. The local recurrence and distant metastasis rates were 42.9% and 19.0%, respectively. Therefore, if the tumor is a high grade myxofibrosarcoma or cannot be completely removed, additional radiotherapy and chemotherapy should be considered. However, the rarity of this tumor is an obstacle in establishing effective chemotherapy regimens and radiotherapy doses. In our case, although we prevented sudden death or embolic event, the patient died after 3 months because of incomplete surgical resection and not performing early additional radiotherapy and/or chemotherapy.

• #33 Prognostic Factors and Outcomes of Patients with Myxofibrosarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3837421/

  Distant metastatic disease occurred in 11 patients (16 %) at a median of 10 months (range 364 months) after diagnosis, including 3 patients (4 %) who also developed local recurrences. […] Tumor size (HR 1.16, P = 0.004) and receipt of chemotherapy (HR 3.50, P = 0.048) were the only univariately significant predictors of distant metastatic disease.

• #34

  https://link.springer.com/article/10.1007/s004280000327

  Angiogenesis is an important factor in the morphological progression and metastasis of many solid tumours. […] Cases of MFS varied histologically from hypocellular, mainly myxoid, neoplasms (low-grade malignant, 18 cases) to intermediate-grade malignant lesions with increased cellularity and mitotic activity (13 cases), and high-grade malignant cases with marked pleomorphism, high proliferative activity and areas of necrosis in many cases (12 cases). […] The average IMD in intermediate and high-grade malignant MFS (4.03 and 4.09, respectively) was significantly higher than in low-grade malignant MFS (2.73). […] In conclusion, morphologic tumour progression in MFS is associated with increased IMD, whereas, in MRLS, no such correlation is seen.

• #35 Undetected Myxofibrosarcoma in a Patient Diagnosed with Long Standing Complex Regional Pain Syndrome; A Case Report | Surgical Case Reports | Science Repository | Open Access

  https://www.sciencerepository.org/undetected-myxofibrosarcoma-in-a-patient-diagnosed-with-long-standing-complex-regional-pain-syndrome-a-case-report_SCR-2019-6-108

  Five months after the amputation the patient reported pain in the residual limb that prevented her from using the prosthesis. […] Together with the patient and her family it was decided to have radiotherapy as palliative treatment. […] The usual treatment for MFS is a limb salvage excision accompanied by perioperative local radiotherapy. […] However, MFS exhibits an aggressive loco-regional behavior, and with the limb salvage treatment local recurrence (LR) rates are high (16-31% in 3.5 years), as well as secondary amputation for LR treatment (17-20%). […] Mitotic activity and tumor necrosis are predictors for metastatic recurrence, and increasing tumor size, histologic grade, and tumor attachment to bone have been suggested as negative prognostic indicators. […] The association between CRPS-I and malignancies is unclear. […] However, we hypothesize that in this particular case the CRPS-I and the neoplasm were independent events.

• #35 Undetected Myxofibrosarcoma in a Patient Diagnosed with Long Standing Complex Regional Pain Syndrome; A Case Report | Surgical Case Reports | Science Repository | Open Access

  https://www.sciencerepository.org/undetected-myxofibrosarcoma-in-a-patient-diagnosed-with-long-standing-complex-regional-pain-syndrome-a-case-report_SCR-2019-6-108

  A soft tissue lesion was detected on the affected limb and she was sent back to her primary hospital for further study of this lesion and exclusion of an alternative diagnosis. […] Post-operative histology of the amputated limb revealed a myxofibrosarcoma, a rare malignant tumor. […] The patient died from metastatic complications 17 months post-amputation. […] The most important take away lessons in this case report are the importance of never discarding a neoplasm in presence of a growing mass even in the case of other possible causes and the fact that it merits a thorough diagnostic procedure, as well as the recommendation to conduct diagnostic tests in reference hospitals specialized in the relevant techniques. […] The pathologic analysis of the limb revealed a tumor, a grade 2 MFS with invasion of muscle and bone tissue, with a maximum diameter of 7cm.

• #36 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  In contrast to the findings of previous studies, which described superficial tumors as having no substantial impact on recurrence, our study revealed that superficial tumors were associated with a heightened probability of recurrence. […] In this study, we found that the likelihood of recurrence in patients who underwent planned surgery was significantly lower than that in patients who underwent unplanned surgery. […] Our analysis revealed that serum CK levels were notably lower in patients who subsequently experienced relapses than in those who did not. Furthermore, subsequent multivariate analysis established low CK levels as an independent prognostic factor for MFS recurrence. […] Superficial lesions are prone to infiltration, in contrast to deep lesions, which typically manifest as a singular, discrete masses with a nodular appearance, that extends longitudinally. […] The absence of histologic findings that could be instrumental in predicting local recurrence further complicates the therapeutic approach.

• #36 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  Myxofibrosarcoma (MFS) is a malignant fibroblastic/myofibroblastic neoplasm with a prominent myxoid area. It has the clinical features of frequent local recurrence (LR) and occasional distant metastasis. […] LR was observed in 25 patients (48.08%), and 4 patients developed metastasis. A worse LR rate was found for patients with a low CK level (84.21%) than for those with a high CK level (27.27%) at 5 years (p<0.05). The LR rate of patients who underwent planned surgery was lower than that of patients who underwent unplanned surgery (p<0.05). [...] The probability of LR in patients with MFS was significantly greater in association with unplanned operations, positive margins, low serum CK levels or superficial tumor depth. [...] The time to LR was observed to vary between 18 and 27 months, although instances of LR have been documented up to 8 years following initial treatment. The LR rate within the studied population was 48%, and multiple recurrences have been recorded in a substantial number of patients.

• #37 Impact Of Myxofibrosarcoma On Quality Of Life – Klarity Health Library

  https://my.klarity.health/impact-of-myxofibrosarcoma-on-quality-of-life/

  Patients with myxofibrosarcoma often experience significant physical symptoms due to tumour growth. Pain and discomfort are common, arising from tumour pressure on nerves and surrounding tissues. These symptoms can severely limit mobility and physical functioning, restricting the ability to perform daily activities and diminishing overall well-being. […] The physical limitations imposed by both the disease and its treatment significantly impact patients’ ability to perform routine tasks. Many patients experience a degree of dependence on caregivers for assistance with daily activities, which can diminish their sense of independence and self-worth. This dependence often leads to a reduced quality of life, as patients struggle to adapt to new limitations and roles within their daily lives.

• #38 Impact Of Myxofibrosarcoma On Quality Of Life – Klarity Health Library

  https://my.klarity.health/impact-of-myxofibrosarcoma-on-quality-of-life/

  The diagnosis and ongoing management of myxofibrosarcoma are associated with considerable emotional stress. Patients frequently experience anxiety related to the fear of recurrence or metastasis, which can overshadow their day-to-day lives. Additionally, the anticipation of medical procedures and treatments can lead to heightened anxiety, further impacting mental health. […] Chronic pain, physical limitations, and the psychological burden of living with cancer can lead to significant mental health challenges. Many patients with myxofibrosarcoma experience depression, exacerbated by the ongoing struggles with their physical health, and the existential threat posed by their diagnosis. […] Physical changes resulting from surgery, such as scarring and disfigurement, can profoundly affect body image and self-esteem. Patients may feel socially isolated due to their altered appearance, which can lead to withdrawal from social activities and support networks. This social isolation further compounds the psychological burden and diminishes quality of life.

• #39 Myxofibrosarcoma symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/myxofibrosarcoma

  Myxofibrosarcoma is a type of soft tissue sarcoma related to malignant fibrous histiocytomas. It is associated with a greater risk of local recurrence than other soft tissue sarcomas. […] Common symptom Pain […] 2 myxofibrosarcoma patients report severe pain (66%) […] Common symptom Fatigue […] 1 a myxofibrosarcoma patient reports severe fatigue (33%) […] Common symptom Stress […] 1 a myxofibrosarcoma patient reports moderate stress (100%) […] Common symptom Anxious mood […] 2 myxofibrosarcoma patients report moderate anxious mood (66%) […] Common symptom Depressed mood […] 2 myxofibrosarcoma patients report moderate depressed mood (66%).

• #40 Reddit – The heart of the internet

  https://www.reddit.com/r/sarcoma/comments/1epkqo5/myxofibrosarcoma_surgery_this_week/

  Ive been diagnosed with Myxofibrosarcoma in my leg and am getting it surgically removed next week. The wound is going to require skin grafts and also tissue and muscle transplants to fill the gap in my leg. The wound itself is huge, I think about 8 inches minimum and Im pretty small. The doctors havent been clear what life will look like post surgery. […] Im told Ill always have an indent/disfigurement of my leg. Will I have chronic pain? Will I ever be able to do sports again? Im just not sure what my quality of life will be post-operation. How is the recover from the donor sites (other leg and back?)

• #41 A Second Opinion Changed Joel’s Grade 2 Myxofibrosarcoma Treatment Plan – The Patient Story | For Cancer Patients & Caregivers

  https://thepatientstory.com/patient-stories/sarcoma/soft-tissue/myxofibrosarcoma/joel-s/

  After radiation, surgeons will remove the tumor and perform a skin graft and muscle repositioning to help the area heal properly. […] His belief in God provides reassurance, removing the fear of death and allowing him to focus on living fully. […] He considers how his initial reluctance to get checked could have caused worse outcomes. […] Now, he encourages others to listen to their bodies and take proactive steps.

• #41 A Second Opinion Changed Joel’s Grade 2 Myxofibrosarcoma Treatment Plan – The Patient Story | For Cancer Patients & Caregivers

  https://thepatientstory.com/patient-stories/sarcoma/soft-tissue/myxofibrosarcoma/joel-s/

  Joel has always valued family, faith, and community. […] However, in a life-changing moment, in December 2024, at age 57, he was diagnosed with a grade 2 myxofibrosarcoma, a rare soft tissue cancer. […] A small lump on his shin seemed minor at first, and he initially dismissed it as a fatty growth. However, over several months, it grew larger, prompting him to visit his doctor. […] The uncertainty of his diagnosis was overwhelming. Not knowing whether he had months or years left to live was a heavy burden. […] However, after a biopsy confirmed it was grade 2 myxofibrosarcoma and contained, he felt a sense of relief. […] Joel’s grade 2 myxofibrosarcoma treatment plan consists of radiation therapy, which he undergoes daily for a few minutes. […] Though he hasn’t experienced significant side effects yet, doctors have advised him that he may develop skin irritation in later weeks.

• #42 Impact Of Myxofibrosarcoma On Quality Of Life – Klarity Health Library

  https://my.klarity.health/impact-of-myxofibrosarcoma-on-quality-of-life/

  The physical and psychological challenges associated with myxofibrosarcoma often lead to an inability to work, resulting in a loss of income. The financial burden of treatment and ongoing care can be substantial, placing additional stress on patients and their families. This economic strain further diminishes quality of life, as financial insecurity adds to the existing challenges. […] In summary, myxofibrosarcoma significantly impacts patients’ quality of life across physical, psychological, and social dimensions. The physical burden of symptoms and treatment side effects, coupled with the psychological stress and social challenges, underscores the importance of a comprehensive and multidisciplinary approach to care. Effective management and support strategies, informed by ongoing research and tailored to the unique needs of myxofibrosarcoma patients, are essential for enhancing quality of life.

• #43 Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9244941/

  The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. […] Since local recurrence frequently occurs but distant metastasis is rare, the current standard of care for localized disease is radical resection confirmed by histological clear surgical margins combined with neoadjuvant or adjuvant radiotherapy. […] Despite treatments, local recurrence rates are between 50% and 60%. […] Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. […] Typically, the first-line chemotherapeutic treatment for recurrent and metastatic MFS includes anthracycline (doxorubicin) and ifosfamide, either alone or in combination, however its response rates in advanced STS are about 20-30%.

• #44 A Case of Myxofibrosarcoma in the Maxillary Sinus

  https://www.kjorl.org/journal/view.php?number=8782

  Surgery is the primary treatment for MFS. […] Adjuvant RTx can be used where the tumor is unresectable or large, the margin after resection is close to within 1 cm, the margin is positive or the tumor is histologically high-grade. […] The patient has been under surveillance for a period of 2 years, during which no signs of recurrence have been detected.

• #45

  https://crimsonpublishers.com/tnn/fulltext/TNN.000530.php

  A wide margin surgical resection aimed at achieving negative margins is the main modality of treatment of Myxofibrosarcomas. Radiation is an effective adjuvant treatment in these patients and should be considered in the local management of this disease. […] Myxofibrosarcoma of skull present a challenging management conundrum owing to the combination of exceedingly rare occurrence, varied pathologic features, infiltrative tendency, high local recurrence rate and stage progression in recurrences.

• #46 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  They first did 6 weeks of radiation then surgery. It came back in 3/2017 and they had to amputate his left leg right below the knee. Now its come back again but in his lungs (8/2018). […] The stage is at iv, and she refused to be admitted to any hospital when the doctor said she had serious infection. […] Basically, he told her more or less, that the radiation is just maintenance therapy. There is not much else.

• #47 A novel patient-derived immortalised cell line of myxofibrosarcoma: a tool for preclinical drugs testing and the generation of near-patient models | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-11658-9

  Myxofibrosarcoma is a rare malignant soft tissue sarcoma characterised by multiple local recurrence and can become of higher grade with each recurrence. Consequently, myxofibrosarcoma represents a burden for patients, a challenge for clinicians, and an interesting disease to study tumour progression. MFS typically arises as a painless and slow-growing mass with infiltrative margins affecting the extremities. Histologically, MFS is characterised by atypical spindle cells immersed in a gelatinous myxoid matrix with a tendency of multinodular and lobular growth and with curvilinear vessels. The major issue is that after several relapses and surgical interventions, patients may undergo amputation. These clinical challenges make it necessary to deepen the knowledge about MFS neoplastic progression and response to conventional or innovative treatments. Results demonstrate that these cells can close the gap in 44 hours, and even if the MF-R 3 wound closure rate is inferior compared to 143B cells it is still remarkable. Moreover, MF-R 3 cells can migrate through a collagen-coated transwell membrane showing strong invasive potential, notably reaching similar results to 143B cells. The results of 2D and 3D clonogenic potentials indicate a staminal nature and the ability to replicate and form a growing mass even from a very low cell number and in challenging conditions. This great in vitro invasive potential reflects the highly invasive and metastatic nature of the tumour of origin indicated by the presence of vertebral metastases.

• #48 Combination nivolumab and bevacizumab for metastatic myxofibrosarcoma: A case report and review of the literature

  https://www.spandidos-publications.com/10.3892/mco.2020.2124

  The main therapeutic strategy for metastatic Myxofibrosarcoma (MFS) is palliative chemotherapy. […] The most frequent adverse events were fatigue, abnormality of Alanine aminotransferase (ALT), hypertension and proteinuria. […] The predominant side effects of the treatment included fatigue, elevated ALT levels, hypertension and proteinuria. […] The patient obtained continuous disease control and long-term survival benefits. […] The primary side effects included fatigue, abnormal ALT levels, hypertension and proteinuria, which were all safe and controllable.

• #49 Myxofibrosarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/myxofibrosarcoma/

  Myxofibrosarcoma (MFS) is a rare type of soft tissue sarcoma known for its high local recurrence rates. It is characterized by an infiltrative growth pattern, which contributes to its tendency to recur locally. This type of cancer is more likely to return after treatment compared to other soft tissue sarcomas, with up to 50% of patients experiencing recurrence within five years. […] Due to the high recurrence rate of MFS, regular follow-up care is essential. Patients undergo routine imaging to monitor for any signs of recurrence, which allows for early intervention if the cancer returns. This proactive approach is crucial in managing MFS and improving long-term outcomes. […] Local recurrence is a significant concern for MFS patients. The five-year local recurrence-free survival rate is about 72%. Recurrence is often more aggressive, and the median overall survival after local recurrence is approximately 64 months.

• #50 Myxofibrosarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/myxofibrosarcoma/

  Living with MFS involves regular follow-up care to monitor for recurrence. Regular imaging and consultations with healthcare providers are essential to detect and treat any recurrence early. Patients are encouraged to maintain open communication with their healthcare team to manage the disease effectively. The treatment plan aims to eliminate all cancer cells to reduce the likelihood of recurrence.

• #51 Fibrosarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/fibrosarcoma/

  Symptoms of fibrosarcoma can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with fibrosarcoma: […] A lump or swelling in the soft tissue of the body under the skin, which can occur anywhere in the body […] This lump can be fast or slow-growing, and can be painful. […] Fibrosarcoma can reappear in the same area after the treatment of a previous tumour; this is called a local recurrence. […] A recurrence of fibrosarcoma may be accompanied by cancer in other parts of the body. This is called metastasis or secondary cancer. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary fibrosarcoma tumour. In fibrosarcoma patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments. […] Secondary cancers may also appear in the bone and lymph nodes.

• #52 Myxofibrosarcoma diagnosis – GPs slow to recognise? – Macmillan Online Community

  https://community.macmillan.org.uk/cancer_types/soft-tissue-sarcomas-forum/f/diagnosis-and-treatment/281165/myxofibrosarcoma-diagnosis—gps-slow-to-recognise

  In May 2024 I was diagnosed with low grade myxofibrosarcoma in my right arm after more than three years of baffled doctors looking at my slowly-swelling right forearm with visible bruising. […] Given the time it has taken to get a diagnosis, my tumour has progressed to more than 32cm length when it started off as a very localised swelling at my elbow. […] I was pregnant at the time and was told this was pregnancy swelling in my thigh. […] 18 month later and here I am diagnosed with Myxoid Liposarcoma.

• #53 Low-grade myxofibrosarcoma | Radiology Case | Radiopaedia.org

  https://radiopaedia.org/cases/low-grade-myxofibrosarcoma-1?lang=us

  One month ago, the patient noticed a swelling of the right thigh. No pain nor any other symptoms. […] The surgical specimen showed invasion into the femoral canal consistent with the MRI findings showing less well-defined fascia and edema focally extending into the femoral canal. This shows the importance of MR-graphic assessment since sonographic evaluation did not allow visualization of the extension into the femoral canal. […] Pathologically proven low-grade myxofibrosarcoma with all the typical features: patient age over 60, painless mass, located in the lower limb which is the most prevalent area for this lesion, high T2 / low T1 signal consistent with myxoid tissue, partial enhancement since only the solid (not myxoid) components enhance.

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